Antiphospholipid antibody syndrome
Section editors | |||
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Shruti Chaturvedi, MBBS, MSCI Baltimore, MD |
Benjamin Tillman, MD Nashville, TN |
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1 variants on this page
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Guidelines
To be completed. Due to the paucity of trials in this particular condition, many seek guidance from the venous thromboembolism (VTE) literature.
All lines of therapy
Warfarin monotherapy
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Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Crowther et al. 2003 | Phase III (C) | High-intensity Warfarin (INR 3.1 to 4.0) | Seems not superior |
Finazzi et al. 2005 (WAPS) | Phase III (C) | High-intensity Warfarin (INR 3.0 to 4.5) | Seems not superior |
Cohen et al. 2016 (RAPS) | Phase II/III (C) | Rivaroxaban | Inconclusive whether non-inferior |
Pengo et al. 2018 (TRAPS) | Phase III (C) | Rivaroxaban | Fewer events |
Note: TRAPS was closed prematurely due to excess events in the rivaroxaban arm.
Anticoagulation
- Warfarin (Coumadin) PO titrated to goal INR 2.0 to 3.0
- Note: some trials specify a goal INR 2.5 without a range given
Continued indefinitely
References
- Crowther MA, Ginsberg JS, Julian J, Denburg J, Hirsh J, Douketis J, Laskin C, Fortin P, Anderson D, Kearon C, Clarke A, Geerts W, Forgie M, Green D, Costantini L, Yacura W, Wilson S, Gent M, Kovacs MJ. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003 Sep 18;349(12):1133-8. Erratum in: N Engl J Med. 2004 Jul 8;351(2):200. N Engl J Med. 2003 Dec 25;349(26):2577. link to original article PubMed
- WAPS: Finazzi G, Marchioli R, Brancaccio V, Schinco P, Wisloff F, Musial J, Baudo F, Berrettini M, Testa S, D'Angelo A, Tognoni G, Barbui T. A randomized clinical trial of high-intensity warfarin vs conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS). J Thromb Haemost. 2005 May;3(5):848-53. link to original article PubMed
- RAPS: Cohen H, Hunt BJ, Efthymiou M, Arachchillage DR, Mackie IJ, Clawson S, Sylvestre Y, Machin SJ, Bertolaccini ML, Ruiz-Castellano M, Muirhead N, Doré CJ, Khamashta M, Isenberg DA; RAPS trial investigators. Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2/3, non-inferiority trial. Lancet Haematol. 2016 Sep;3(9):e426-36. link to original article link to PMC article PubMed
- TRAPS: Pengo V, Denas G, Zoppellaro G, Jose SP, Hoxha A, Ruffattia A, Andreoli L, Tincani A, Cenci C, Prisco D, Fierro T, Gresele P, Cafolla A, De Micheli V, Ghirarduzzi A, Tosetto A, Falanga A, Martinelli I, Testa S, Barcellona D, Gerosa M, Banzato A. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018 Sep 27;132(13):1365-1371. Epub 2018 Jul 12. link to original article PubMed
Rivaroxaban monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Cohen et al. 2016 (RAPS) | Phase II/III (E) | Warfarin | Inconclusive whether non-inferior |
Pengo et al. 2018 (TRAPS) | Phase III (E) | Warfarin | Higher rate of events |
Note: TRAPS was closed prematurely due to excess events in the rivaroxaban arm. Although we include here for historical context, this regimen should not be used outside of the context of a clinical trial.
Anticoagulation
- Rivaroxaban (Xarelto) PO 20 mg PO once per day
Continued indefinitely
References
- RAPS: Cohen H, Hunt BJ, Efthymiou M, Arachchillage DR, Mackie IJ, Clawson S, Sylvestre Y, Machin SJ, Bertolaccini ML, Ruiz-Castellano M, Muirhead N, Doré CJ, Khamashta M, Isenberg DA; RAPS trial investigators. Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2/3, non-inferiority trial. Lancet Haematol. 2016 Sep;3(9):e426-36. link to original article link to PMC article PubMed
- TRAPS: Pengo V, Denas G, Zoppellaro G, Jose SP, Hoxha A, Ruffattia A, Andreoli L, Tincani A, Cenci C, Prisco D, Fierro T, Gresele P, Cafolla A, De Micheli V, Ghirarduzzi A, Tosetto A, Falanga A, Martinelli I, Testa S, Barcellona D, Gerosa M, Banzato A. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018 Sep 27;132(13):1365-1371. Epub 2018 Jul 12. link to original article PubMed